Genetics Education and Health Research, Murdoch Childrens Research Institute, Melbourne, Victoria, Australia.
Eur J Hum Genet. 2010 Oct;18(10):1084-9. doi: 10.1038/ejhg.2010.78. Epub 2010 May 26.
Newborn screening (NBS) for cystic fibrosis (CF) provides the opportunity for cascade carrier testing of relatives. Uptake of testing by adult non-parent relatives of children diagnosed with CF through NBS has not been previously described, and this study describes uptake by both parents and adult non-parent relatives in Victoria, Australia. Pedigrees were taken from parents of children who were born in 2000-2004 and diagnosed with CF. A total of 40 families were eligible for the study and 30 (75%) were recruited. In all, 716 non-parent relatives were identified from the pedigrees as eligible for carrier testing, and 82 (adjusted uptake percentage: 11.8%; 95% confidence interval 8.0-15.7) have had carrier testing by March 2009. On average, 2.7 non-parent relatives per family had CF carrier testing after diagnosis through NBS. The odds of being tested were greater for females than males (adjusted odds ratio 1.61; 95% confidence interval 1.11-2.33; P=0.01) and greater for those more closely related to the child with CF (adjusted odds ratio 5.17; 95% confidence interval 2.38-11.24; P<0.001). Most relatives who undergo testing are tested immediately after the baby's diagnosis; however, some testing is undertaken up to 8 years later. These results indicate that in a clinical setting, the diagnosis of a baby with CF by NBS does not lead to carrier testing for the majority of the baby's non-parent relatives. We suggest re-contact with parents to offer cascade carrier testing.
新生儿筛查(NBS)为囊性纤维化(CF)提供了对亲属进行级联携带者检测的机会。通过 NBS 对被诊断为 CF 的儿童的成年非父母亲属进行检测的接受程度以前尚未描述过,本研究描述了澳大利亚维多利亚州的父母和成年非父母亲属的接受程度。系谱来自于 2000-2004 年出生并被诊断为 CF 的儿童的父母。共有 40 个家庭符合研究条件,其中 30 个(75%)被招募。总共从系谱中确定了 716 名符合携带者检测条件的非父母亲属,截至 2009 年 3 月,其中 82 人(调整后的接受率:11.8%;95%置信区间为 8.0-15.7)进行了携带者检测。平均而言,每个家庭有 2.7 名非父母亲属在通过 NBS 诊断后接受 CF 携带者检测。女性接受检测的可能性大于男性(调整后的优势比为 1.61;95%置信区间为 1.11-2.33;P=0.01),与 CF 患儿关系越密切的亲属接受检测的可能性越大(调整后的优势比为 5.17;95%置信区间为 2.38-11.24;P<0.001)。大多数接受检测的亲属是在婴儿确诊后立即接受检测的;但是,有些检测是在 8 年后进行的。这些结果表明,在临床环境中,通过 NBS 诊断婴儿患有 CF 并不会导致大多数婴儿的非父母亲属进行携带者检测。我们建议与父母重新联系,以提供级联携带者检测。